Abstract

To estimate the prevalence and significance of cranial nerve (CN) imaging abnormalities in patients with hereditary neuropathy and discuss clinical implications.We retrospectively analyzed data from patients at four tertiary academic medical centers with hereditary neuropathy diagnoses who had undergone gadolinium-enhanced magnetic resonance imaging (MRI) of the brain or skull base between 2004 and 2018. MRI scans, as well as computed tomography imaging when available, were reviewed and bivariable analysis was performed to identify predictors of CN abnormalities on imaging.Among 39 patients meeting study criteria, 11 had clinical CN deficits (28%) and 8 had CN abnormalities on imaging (21%). Of the patients with CN abnormalities on imaging, half had CN deficits (4/8) and only a quarter had imaging abnormalities of the CNs with the deficits (2/8). Imaging abnormalities were found in varied CNs, including CNs III, V, VII, and the VII/VIII complex in the internal auditory canal. MRI obtained for the purpose of evaluating CN deficits had a statistically significant increased likelihood of containing CN imaging abnormalities. However, CN deficits themselves were not predictive of imaging abnormalities.Thickening and enhancement of CNs on MRI may be found in approximately 1/5 of patients with hereditary neuropathies and are inconsistently associated with clinical deficits. These imaging findings should not be mistaken for neoplastic and infectious processes as they may be manifestations of the patients' underlying genetic neuropathy.4.

Effects of Pregnancy on Otosclerosis.Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck SurgeryQian, Z. J., Alyono, J. C.2020: 194599820907093

Abstract

The effect of pregnancy on otosclerosis is controversial. If pregnancy physiologically increases the risk of progression, females with children would be expected to receive stapedectomy earlier than childless females and males. Here, we seek to determine whether sex moderates the relationship between number of children and age at stapedectomy.Retrospective observational study of national health care claims.2003 to 2016 Optum Clinformatics Data Mart.In total, 6025 privately insured US adults (3553 females, 2472 males) who received stapedectomy for otosclerosis were queried for age and number of children at the time of initial surgery.The average age at stapedectomy was significantly lower in females than males (46.8 vs 48.1 years; t test, P < .0001). Females with children had a significantly lower age at surgery compared to childless females (39.3 vs 49.9 years; t test, P < .0001). Males with children similarly had a significantly lower age at surgery compared to childless males (40.5 vs 51.3 years; t test, P < .0001). A higher number of children was correlated with lower age for both females (Pearson, r = -0.3817, P < .0001) and males (Pearson, r = -0. 3675, P < .0001). Linear regression showed that younger age of surgery could be predicted by female sex and number of children (F(3, 6021) = 336.93, P < .001, R2 = 0.1437) with no significant interaction between sex and number of children (P = .186).Sex does not moderate the effect of increasing number of children on decreasing age at stapedectomy. Social, rather than biological, factors surrounding parenthood such as increased overall health care utilization may explain prior associations between pregnancy and otosclerosis.

Abstract

The aim of the study is to examine trends in the age of patients receiving cochlear implants and to determine the effect of age on the rate of perioperative complications.Retrospective analysis of deidentified administrative claims data from a US commercial insurance database (Optum).Individuals undergoing cochlear implantation between 2003 and 2016.US hospital and outpatient facilities serving commercially insured patients.Cochlear implantation.Age at implantation, incidence of perioperative complications within 30 days identified by ICD9/10 codes including device problems, myocardial infarction, stroke, venous thromboembolism, local infection, meningitis, stroke, cerebrospinal fluid leak, and facial weakness.Between 2003 and 2016, 3420 patients underwent a total of 4154 cochlear implants. The number of implants per year increased annually from 171 in 2003 to 531 in 2016, with the greatest growth demonstrated in those aged 60 and older.The age of patients undergoing implantation increased annually from an average of 26.6-57.2 years (p<0.001). The implantation rates from 2003 to 2016, per 100,000 enrollees, increased from 1.64 to 6.82 for patients 60-79 years of age, and 0 to 11.57 for patients greater than 80 years of age (p<0.001). No significant differences in 30-day complication rates were found between patients when grouped by age in decades, except for device related problems, which was significantly higher in younger patients (<18 years).Over the past decade and a half, cochlear implantation is more frequently being performed, and in an increasingly aging population. This trend does not seem to alter the risk of perioperative complications.

Abstract

OBJECTIVE: To evaluate the clinical presentation and management strategies for neonatal retropharyngeal abscess (RPA).METHODS: Retrospective chart review was performed, and literature reviewed.RESULTS: We report two cases of neonatal RPA, with one complicated by cervical osteomyelitis, and the other presenting with apparent life-threatening events (ALTEs). A 6-week-old female underwent transoral drainage of an RPA, which grew methicillin sensitive Staphylococcus aureus. She had a prolonged recovery course and was found to have developed osteomyelitis of the dens and atlas. She was treated with 14 weeks of IV antibiotics and rigid collar fixation for spinal cord instability. A 2-month-old female was admitted after multiple ALTEs with episodes of apnea and pallor. Direct laryngoscopy revealed a bulging RPA, which was drained transorally. This grew multiple organisms including methicillin resistant Staphylococcus aureus, Streptococcal oralis and Prevotella species.CONCLUSIONS: Uncommon in neonates, RPA can present in this age group without fever, and are is likely to have airway complications than in older children. In cases with prolonged recovery, additional diagnostic intervention is recommended to rule out rare complications such as osteomyelitis. Emphasis in such complex cases is placed on a multidisciplinary approach to patient care, coordinating neonatologists, infectious disease specialists, neurosurgeons, and otolaryngologists.

Abstract

OBJECTIVE: To determine the incidence of postoperative venous thromboembolism (VTE) in adults undergoing otologic surgery.STUDY DESIGN: Cross-sectional retrospective study.SETTING: Single tertiary academic center.SUBJECTS AND METHODS: Adults undergoing nononcologic, extracranial otologic surgery from August 2009 to December 2016. Patients with postoperative diagnosis VTE codes were identified. Imaging and clinical documents were searched for VTE evidence within the first 30 postoperative days. Methods of thromboprophylaxis were documented, and Caprini risk scores were calculated.RESULTS: In total, 1213 otologic surgeries were evaluated. No postoperative VTE events were identified (0/1268). Mean age was 51.0 17.3 years (range, 18.1-93.4 years). Average length of surgery was 136.0 79.0 minutes (range, 5-768 minutes). The average Caprini score in all patients was 4.0 1.7 (range, 1-15). Eighty-five percent of patients had a Caprini score 3, the threshold at which chemoprophylaxis has been recommended in general surgery patients by the American College of Chest Physicians 2012 guidelines. Six patients had documented preoperative chemoprophylaxis and a Caprini score of 4.8 1.7. This was not significantly different from that of patients who did not receive preoperative chemoprophylaxis ( t test, P = .3). The literature would estimate a rate of 3.7% VTE in adults with similar Caprini scores undergoing general surgery procedures with no VTE prophylaxis.CONCLUSION: The Caprini risk assessment model may overestimate VTE risk in patients undergoing extracranial otologic surgery. Postoperative VTE following otologic surgery is rare, even in patients traditionally considered moderate or high risk. Chemoprophylaxis guidelines in this group should be balanced against the potential risk of increased intraoperative bleeding and its associated effects on surgical visualization and morbidity.

Abstract

To prospectively evaluate opioid consumption following adult outpatient otologic surgery.Prospective observational.Single-tertiary referral center.Patients scheduled for otologic surgery who did not have a history of chronic opioid use were recruited between February 2018 and February 2019.Opioid consumption was queried using telephone or in-person surveys administered between postoperative days 5 and 15. Patient demographics, surgical details, and opioid prescription patterns were abstracted from medical records. Opioid distribution was determined by querying records maintained by the California Department of Justice through a state-wide prescription drug monitoring program mandated since 2016.Seventy patients were prescribed an average of 68.931.8mg of morphine equivalents (MME) and consumed 47.342.9 MME over 2.42.3 days postoperatively. Patients who received a postauricular incision were prescribed significantly more than those who underwent transcanal procedures (86.2 vs 55.9 MME; t test, p<0.001), consumed significantly more (72.2 vs 28.6 MME; t test, p<0.001), and for a significantly longer duration (3.4 vs 1.6 days; t test, p=0.001). In the postauricular group, there was no significant difference in consumption between mastoidectomy and nonmastoidectomy subgroups (64.9 vs 89.2 MME; t test, p=0.151). Eighty percent of transcanal patients consumed 50 MME (10 pills) or less, while 80% of postauricular patients consumed 80 MME (16 pills) or less.Patients in our cohort consumed approximately 3/4 of the prescribed opioids. Those with postauricular incisions used significantly more than those with transcanal incisions. Postoperative opioid prescription recommendations should be tailored according to the extent of surgery.

An association between marijuana use and tinnitus.American journal of otolaryngologyQian, Z. J., Alyono, J. C.2019: 102314

Abstract

While some advocates have argued for marijuana as a treatment for tinnitus, the relationship between marijuana use and tinnitus is unknown. The objective of this study was to evaluate associations between marijuana use and the prevalence, severity, and rate of occurrence of tinnitus.Cross-sectional analysis of nationally representative data.National Health and Nutrition Examination Survey 2011-2012.Statistical analysis was performed on data collected from 2705 non-institutionalized adults aged 20-69 who underwent audiometric testing and were administered questionnaires about hearing, drug use, current health status, and medical history.The use of marijuana at least once per month for the previous 12months was significantly associated with experiencing tinnitus during that 12-monthmonth (X2(1)=19.41, p<0.001). Subjects who used marijuana were more likely to experience tinnitus after accounting for covariables including age, gender, audiometric hearing loss, noise exposure history, depression, anxiety, smoking, salicylate use, cardiovascular disease, hypertension, and diabetes (OR=1.75, 95% CI 1.02-3.01, p=0.043). There were no associations between the severity or frequency of tinnitus occurrence and the quantity or frequency of marijuana use. Use of other substances such as alcohol, cocaine, methamphetamine, and heroin was not associated with tinnitus.Regular marijuana use is associated with prevalent tinnitus. However, no dose response between marijuana use and tinnitus was observed. The relationship between marijuana use and tinnitus is complex and is likely modulated by psychosocial factors.

Abstract

OBJECTIVE: To determine the prevalence of radiographic cochlear-facial nerve dehiscence (CFD).STUDY DESIGN: Retrospective radiological study.SETTING: Two tertiary-referral centers.PATIENTS: Two hundred six temporal-bone computed tomography (CT) scans (405 total ears) of otology/neurotology patients from two academic institutions between the years 2014 and 2017.INTERVENTION: Diagnostic.MAIN OUTCOME MEASURES: The cochlear-facial nerve partition width (CFPW) was measured on coronal CT sections and defined as the shortest distance between the cochlear basal turn and facial nerve (FN) labyrinthine segment. We used logistics regression analyses to determine positive predictors for radiographic evidence of CFD.RESULTS: The overall prevalence of radiographic CFD was 5.4% (22/406 ears). 9.2% of patients (19/206) had CFD. Of these 19 patients, only one patient had mixed hearing loss that could not be explained by any other vestibular or auditory etiology. Three out of 206 patients had dehiscence in both ears (1.4%). The average CFPW was 0.60.2mm, and fallopian canal width was 1.10.02mm (n=405). Older age, use of traditional CT scans, and thinner CT slice thickness were significant predictors for radiographic CFD.CONCLUSIONS: The radiographic prevalence of CFD is higher than what is reported in histologic studies, and may over-estimate the true prevalence of CFD. The clinician should keep this in mind when considering this as potential cause for third-window symptoms.

Abstract

BACKGROUND: Noise-induced hearing loss is one of the most common occupational hazards in the United States. Several studies have described noise-induced hearing loss in patients following mastoidectomy. Although otolaryngologists care for patients with noise-induced hearing loss, few studies in the English literature have examined surgeons' occupational risk.METHODS: Noise dosimeters and sound level meters with octave band analyzers were used to assess noise exposure during drilling of temporal bones intraoperatively and in a lab setting. Frequency specific sound intensities were recorded. Sound produced using burrs of varying size and type were compared. Differences while drilling varying anatomic structures were assessed using drills from two manufacturers. Pure tone audiometry was performed on 7 to 10 otolaryngology residents before and after a temporal bone practicum to assess for threshold shifts.RESULTS: Noise exposure during otologic drilling can exceed over 100dB for short periods of time, and is especially loud using large diameter burrs > 4mm, with cutting as compared with diamond burrs, and while drilling denser bone such as the cortex. Intensity peaks were found at 2.5, 5, and 6.3kHz. Drilling on the tegmen and sigmoid sinus revealed peaks at 10 and 12.5kHz. No temporary threshold shifts were found at 3 to 6kHz, but were found at 8 to 16kHz, though this did not reach statistical significance.CONCLUSION: This article examines noise exposure and threshold shifts during temporal bone drilling. We were unable to find previous descriptions in the literature of measurements done while multiple people drilling simultaneously, during tranlabyrinthine surgery and a specific frequency characterization of the change in peach that appears while drilling on the tegmen. Hearing protection should be considered, which would still allow the surgeon to appreciate pitch changes associated with drilling on sensitive structures and communication with surgical team members. As professionals who specialize in promoting the restoration and preservation of hearing for others, otologic surgeons should not neglect hearing protection for themselves.

Abstract

Vertigo and dizziness are common conditions among older adults. They are closely associated with fall risk and portend major implications for geriatric injury and disability. Management can be particularly challenging, because symptoms are often nonspecific and may reflect multiple etiologies. Chronic dizziness can reflect dysfunction in the vestibular, somatosensory, or visual systems or in their central integration. Systemic processes, such as postural hypotension, arrhythmias, heart failure, medication use, and lower extremity weakness or frailty, also contribute. Management of acute vestibular syndrome requires ruling out dangerous causes, such as stroke. This article reviews relevant definitions, epidemiology, pathophysiology, diagnosis, and clinical management.

Practice of Otology During the First Quarter Century of the American Otological Society (1868-1893).Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and NeurotologySioshansi, P. C., Jackler, R. K., Alyono, J. C.2018; 39 (4S Suppl 1): S10S29

Abstract

To describe the practice of otology in America during the first quarter century of the American Otological Society (AOS).Two sources were used to determine the most prevalent disease conditions cared for and surgical procedures undertaken during this era. All articles published in the AOS transactions between 1868 and 1893 were studied as were the otology textbooks published by 6 of the first 10 Presidents of the Society.The primary emphasis of late 19th century American otological scholarship was on chronic ear infection with numerous articles focusing on complications of otitis including frequent descriptions of fatalities. Much emphasis was placed upon the Eustachian tube with catheterization and insufflation a major part of otological practice. Due to limitations in technology, the overwhelming focus was on diseases of the ear canal and middle ear. Understanding of temporal bone anatomy was much superior to that of physiology. Erroneous speculations on the function of the middle and inner ear were common. Surgical interventions were largely limited to myringotomy and mastoidectomy, the latter of which was sometimes life saving during the preantibiotic era.The latter half of the 19th century saw the emergence of otology as a specialty in America and many emerging diagnostic and therapeutic advances were adopted. While capabilities were notably limited during this era, the efforts of a small band of pioneer otologists in the founder generation of the AOS contributed greatly to the progress of the emerging specialty.

Abstract

To determine the feasibility of using temporal bone computed tomography (CT) scans to identify malleal ligaments and the prevalence of calcification in malleal ligaments.Retrospective case review. CT scans were blindly and retrospectively reviewed by two physicians (a radiologist and a nonradiologist). Scans differed by slice thickness, and included both conventional CT and cone beam CT (CBCT).Ambulatory tertiary referral center.One hundred fifty-one temporal bone CT scans, obtained between the years 2014 and 2017, were initially screened, which included 302 ears. Patients with previous tympanomastoid surgery or middle ear opacification were excluded, leaving 187 ears in the study.Diagnostic.Percentage of visible normal and calcified malleal ligaments.Scans with submillimeter slice thickness were more likely to demonstrate all three malleal ligaments than those with 1 ml and larger slices (83.7% versus 50.0% for nonradiologist, p<0.0001; 59.6 versus 34.8% for radiologist, p<0.0001). Calcification was seen in 11.8% of ears reviewed. The ability to detect malleal ligaments with cone beam CT was 86.2%, while the rate with conventional CT was 71.1%, a difference that persisted when controlling for slice thickness. Interobserver agreement for the detection of malleal ligaments was 65% with a Cohen's kappa coefficient of =0.27.Visualization of the malleal ligaments using CT scans is feasible in a majority of aerated ears. Detection of malleal ligaments improves with thinner slice thickness and cone-beam technique. Low interobserver agreement suggests the importance of experience and a need for standardized review.

The American Otological Society at its Sesquicentennial: Insights Into the Society's Formative Years.Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and NeurotologyJackler, R. K., Alyono, J. C., Mudry, A. C.2018; 39 (4S Suppl 1): S1S9

Abstract

To elucidate the sequence of events which led to the formation of the American Otological Society (AOS) in 1868 and to examine the lives and contributions of the nine founding members of the Society.Study of primary historical documents, biographical material, and previous histories of the AOS.Earlier treatments of the history of the AOS minimally covered the events and personalities from the Society's formative period. The founders of the AOS were much influenced by recent advances in European Otology and the success of the nascent American Ophthalmological Society which had been founded in 1864. The AOS has long credited Elkanah Williams as its first president of the AOS, although he never actually served in this role and was not a contributor to otological literature. Documents suggest that 30 years old New York physician Daniel Bennett St John Roosa, recently returned from a grand tour of the leading European otological centers, was the principal advocate for the creation of the AOS.The 1860s were a pivotal period in the maturation of American Otology. Previously, most "aurists" were widely considered to be charlatans who practiced unscientifically and often unscrupulously. The AOS founder generation were a group of Ophthalmologists who strove to elevate otology from being a lesser appendage of the mother field to becoming a respected and scientifically based medical specialty in its own right.

Abstract

To describe the history of women in the American Otological Society (AOS).Biographies of the early women of the AOS were compiled through review of the AOS transactions, their published scholarship, newspaper articles, and memorials. Interviews were conducted with the only two women to have led the society and also with former colleagues and family members of pioneering AOS women members who are no longer with us. The evolving gender composition of the society over time was researched from AOS membership lists and compared with data on surgical workforce composition from multiple sources such as the Association of American Medical Colleges, Accreditation Council for Graduate Medical Education, American Medical Association, and the American Academy of Otolaryngology-Head and Neck Surgery.Although American women specialized in otology as far back as 1895, the first woman to be invited to join the AOS as Associate member in 1961 was Dorothy Wolff, PhD. The first female full member was otologic surgeon LaVonne Bergstrom, M.D., who was elected in 1977, 109 years after the foundation of the Society. As of 2017, only two women have served as AOS President. The first was Aina Julianna Gulya, M.D., who took office during the 133rd year in 2001. At the time of the sesquicentennial (2017), 7.5% of AOS members are women including three of eight who serve on the AOS Council. This compares with 15.8% of women among the otolaryngology workforce and a growing 10.9% representation among those who have earned subcertification in neurotology.Gender disparities remain in the AOS, but both participation and scholarly contributions by women in otology have grown substantially since the society's inception 150 years ago, and particularly in the 21st century. Increasing the presence of women in leadership provides role models and mentorship for the future.

Abstract

To describe extracochlear extension of revision cochlear implant arrays into the Fallopian canal.Two adult patients with extension of revision cochlear implant arrays into the Fallopian canal.Computed tomography (CT), selective deactivation of non-functional electrodes.Facial nerve function, facial nerve stimulation, cochlear implant electrode position, radiography, functional hearing.Two patients presented with failure of their long-standing cochlear implants (CIs). One patient with presumed postviral hearing loss presented with declining function and increasing facial stimulation from an implant placed 30 years previous. A second with postmeningitic hearing loss presented with a draining mastoid fistula from an implant placed 7 years before. Both patients were reimplanted with minimal insertion resistance via the established electrode tract, yet demonstrated facial nerve stimulation during intraoperative telemetry and on device activation. Postoperative CTs of each patient showed exit of the electrode from the cochlea into the tympanic or labyrinthine Fallopian canal. Both patients can use their devices effectively with selective electrode deactivation.Our cases illustrate the potential association of long-standing electrodes with otic capsule changes, allowing extracochlear malposition of subsequent arrays. This can occur despite apparently uneventful reinsertion of a flexible array without undue force. Previously reported histopathology confirms the potential for a reactive osteitis from arrays that may contribute to this phenomenon. Intraoperative facial stimulation with neural telemetry testing can raise suspicion of a malpositioned array involving the Fallopian canal. Such cases can be effectively managed with selective deactivation of malpositioned electrode contacts.

Abstract

Kniest dysplasia is an extremely rare form of type II collagenopathy associated with cleft palate, micrognathia, shortened trunk, arms and legs, and club foot. The authors present a case of an infant with this disorder who also had micrognathia and respiratory distress for which mandibular distraction was performed. Although abnormal collagen and impaired endochondral ossification is noted with Kniest dysplasia, adequate bone formation was observed across the distraction gap. Nonetheless, despite stable mandibular advancement, failure to consider concomitant restrictive lung disease resulted in tracheostomy dependence. The authors demonstrate that while successful bone regeneration can be achieved through distraction of intramembranous facial bones, discretion must still be employed in patients with collagenopathies.

Abstract

To describe the presentation, treatment, and outcome of inflammatory pseudotumors (IPs) of the skull base.English-language articles in PubMed, Web of Science, and EMBASE from earliest available through April 2014.Articles were identified using a keyword search for "inflammatory pseudotumor," "inflammatory myofibroblastoma," or "plasma cell granuloma," including a keyword localizing to the skull base.One hundred papers with 157 cases met inclusion criteria. History, tumor site, initial and subsequent treatment, outcomes, and complications were extracted. Student t test, z test, and analysis of variance were used to analyze demographics, symptoms, sites involved, and outcomes. Odds ratios for site versus initial treatment were calculated.At diagnosis, average patient age was 41 years. Approximately 70% of lesions primarily involved the anterior skull base, 29% the lateral skull base, and 1.2% the occiput. The most common initial treatments were steroids (44%), surgery (28%), and surgery with steroids (16%). Anterior lesions were 55.8 times more likely than lateral lesions to be treated initially with steroids (CI, 14.7-212). Seventy-six percent of patients had stable or resolved symptoms after a single course of treatment.Diagnosis of skull base IP requires ruling out other aggressive pathologies, such as malignancy and infection, and maintaining a high index of suspicion. Surgery is favored for lesions that can be removed in toto with minimal morbidity, as well as steroids for those sites where anatomy limits complete resection, such as within the orbit, cavernous sinus, or brain. An option for larger lesions involving vital anatomy is debulking, followed by postoperative steroids.

Abstract

To describe the presentation, treatment, and outcome of inflammatory pseudotumors (IPs) of the skull base.English-language articles in PubMed, Web of Science, and EMBASE from earliest available through April 2014.Articles were identified using a keyword search for "inflammatory pseudotumor," "inflammatory myofibroblastoma," or "plasma cell granuloma," including a keyword localizing to the skull base.One hundred papers with 157 cases met inclusion criteria. History, tumor site, initial and subsequent treatment, outcomes, and complications were extracted. Student t test, z test, and analysis of variance were used to analyze demographics, symptoms, sites involved, and outcomes. Odds ratios for site versus initial treatment were calculated.At diagnosis, average patient age was 41 years. Approximately 70% of lesions primarily involved the anterior skull base, 29% the lateral skull base, and 1.2% the occiput. The most common initial treatments were steroids (44%), surgery (28%), and surgery with steroids (16%). Anterior lesions were 55.8 times more likely than lateral lesions to be treated initially with steroids (CI, 14.7-212). Seventy-six percent of patients had stable or resolved symptoms after a single course of treatment.Diagnosis of skull base IP requires ruling out other aggressive pathologies, such as malignancy and infection, and maintaining a high index of suspicion. Surgery is favored for lesions that can be removed in toto with minimal morbidity, as well as steroids for those sites where anatomy limits complete resection, such as within the orbit, cavernous sinus, or brain. An option for larger lesions involving vital anatomy is debulking, followed by postoperative steroids.

Abstract

Branchial cleft cysts, sinuses, and fistulas are the most common congenital lateral neck lesions in children. They arise as a result of an abnormal development of the branchial arches and their corresponding ectoderm-lined branchial clefts. Of these diverse anomalies, second branchial cleft lesions are the most common, accounting for approximately 95% of all branchial arch pathologies. We describe what is to the best of our knowledge the first reported case of an ectopic tooth in a branchial cleft anomaly. The patient was a young girl who had other congenital abnormalities and syndromic features and who was eventually diagnosed with Townes-Brocks syndrome. We describe the clinical presentation, management, pathologic analysis, and postoperative outcomes of this case, and we present a brief review of Townes-Brocks syndrome.

Abstract

To present a series of patients with facial nerve schwannomas (FNSs) presenting as occluding external auditory canal (EAC) masses.Retrospective case series.Four patients were identified with mastoid segment FNSs occluding the EAC. Three patients presented with conductive hearing loss (CHL), and the fourth presented with facial paralysis, later developing CHL.One patient underwent conservative debulking, removing the EAC component only. Two patients were managed nonoperatively with periodic cleaning of entrapped keratin. The fourth patient received radiation therapy.Facial nerve function, canal cholesteatoma formation, and hearing.Among the patients managed with serial cleaning of entrapped keratin, one maintained normal facial function and one worsened to House-Brackmann II/VI. Facial function worsened to House-Brackmann II/VI in the patient who underwent surgical debulking. The fourth patient, who received radiation, developed complete facial paralysis. All patients accumulated keratin medial to the tumor, and all had CHL.When evaluating an EAC tumor, it is important to obtain imaging before biopsy because biopsy of a schwannoma can result in paralysis. EAC occlusion by a schwannoma presents a challenging management issue, particularly when cholesteatoma forms between the tumor and the tympanic membrane. The primary goal is maintaining normal facial function as long as possible and avoiding secondary ear canal complications. The presence of canal occlusion limits the choice of stereotactic radiation because this leads to a month-long period of tumor swelling and cutaneous sloughing. Resection and grafting are indicated when substantial facial weakness or twitch develops.